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 Formulary Chapter 9: Nutrition and blood - Full Chapter
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09.08.01  Expand sub section  Drugs used in metabolic disorders
Arginine 100mg/mL
(syrup)
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Restricted Drug Restricted
Red
unlicensedUnlicensed

Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders on the advice of tertiary centres in accordance with NHS England commissioning of highly specialised metabolic disorder services.
 
   
Arginine 500mg
(tablet)
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Restricted Drug Restricted
Red
unlicensedUnlicensed

Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders on the advice of tertiary centres in accordance with NHS England commissioning of highly specialised metabolic disorder services.
 
   
Arginine 5g/10mL
(injection)
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Restricted Drug Restricted
Red
unlicensedUnlicensed

Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders, on the advice of the tertiary care centre, in accordance with NHS England commissioning of highly specialised metabolic disorder services. 
   
Biotin 5mg
(tablet)
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Restricted Drug Restricted
Red
unlicensedUnlicensed - Medium Risk

Restricted - to prescribing by Consultant Paediatricians on the recommendation of tertiary Paediatric Specialists only. 
   
Sodium Benzoate 100mg/mL
(oral solution)
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Restricted Drug Restricted
Red
unlicensedUnlicensed

Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders on the advice of tertiary centres in accordance with NHS England commissioning of highly specialised metabolic disorder services.
 
   
Sodium Benzoate 2g/10mL
(injection)
View adult BNF View SPC online View childrens BNF  Track Changes
Restricted Drug Restricted
Red
unlicensedUnlicensed

Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders, on the advice of the tertiary care centre, in accordance with NHS England commissioning of highly specialised metabolic disorder services. 
   
Sodium Benzoate 500mg
(tablet)
View adult BNF View SPC online View childrens BNF  Track Changes
Restricted Drug Restricted
Red
unlicensedUnlicensed

Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders on the advice of tertiary centres in accordance with NHS England commissioning of highly specialised metabolic disorder services.
 
   
Sodium Benzoate 50mg, 250mg, 400mg, 500mg
(capsule)
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Restricted Drug Restricted
Red
unlicensedUnlicensed

Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders on the advice of tertiary centres in accordance with NHS England commissioning of highly specialised metabolic disorder services.
 
   
Strimvelis
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Restricted Drug Restricted
Red Specialist Centre

Use in accordance with NICE HST7 in a specialist centre only.

 
Link  NICE HST7: Strimvelis for treating adenosine deaminase deficiency-severe combined immunodeficiency
   
09.08.01  Expand sub section  Wilsons disease
09.08.01  Expand sub section  Carnitine deficiency
09.08.01  Expand sub section  Fabry's disease
09.08.01  Expand sub section  Gaucher's disease to top
09.08.01  Expand sub section  Mucopolysaccharidosis I
09.08.01  Expand sub section  Pompe disease
09.08.01  Expand sub section  Nephropathic cystinosis
09.08.01  Expand sub section  Urea cycle disorders
Sodium Phenylbutyrate 250mg/mL
(syrup)
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Restricted Drug Restricted
Red
unlicensedUnlicensed

Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders on the advice of tertiary centres in accordance with NHS England commissioning of highly specialised metabolic disorder services. 
   
Sodium Phenylbutyrate 2g/10mL
(injection)
View adult BNF View SPC online View childrens BNF  Track Changes
Restricted Drug Restricted
Red
unlicensedUnlicensed

Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders, on the advice of the tertiary care centre, in accordance with NHS England commissioning of highly specialised metabolic disorder services. 
   
Sodium Phenylbutyrate 500mg (Ammonaps®)
(tablet)
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Restricted Drug Restricted
Red
Restricted - to initiation by Consultant Paediatricians for the treatment of metabolic disorders on the advice of tertiary centres in accordance with NHS England commissioning of highly specialised metabolic disorder services.
 
   
 ....
 Non Formulary Items
Agalsidase Alfa and Beta  (Fabrazyme®)

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Non Formulary
 
Agalsidase Alfa and Beta  (Replagal®)

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Non Formulary
 
Alglucosidase Alfa  (Myozyme®)

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Non Formulary
 
Carglumic Acid  (Carbaglu®)

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Non Formulary
 
Imiglucerase  (Cerezyme®)

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Non Formulary
 
Laronidase  (Aldurazyme®)

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Non Formulary
 
Levocarnitine  (Carnitor®)

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Non Formulary
 
Mercaptamine  (Cystagon®)

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Non Formulary
 
Miglustat  (Zavesca®)

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Non Formulary
 
Sodium Dichloroacetate

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Non Formulary
 
Trientine Dihydrochloride

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Non Formulary
 
Ubidecarenone

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Non Formulary
 
Zinc Acetate  (Wilzin®)

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Non Formulary
 
  
Key
note Notes
Section Title Section Title (top level)
Section Title Section Title (sub level)
First Choice Item First Choice item
Non Formulary Item Non Formulary section
Restricted Drug
Restricted Drug
Unlicensed Drug
Unlicensed
Track Changes
Display tracking information
click to search medicines.org.uk
Link to adult BNF
click to search medicines.org.uk
Link to children's BNF
click to search medicines.org.uk
Link to SPCs
SMC
Scottish Medicines Consortium
Cytotoxic Drug
Cytotoxic Drug
CD
Controlled Drug
High Cost Medicine
High Cost Medicine
Cancer Drugs Fund
Cancer Drugs Fund
NHSE
NHS England
Homecare
Homecare
CCG
CCG

Traffic Light Status Information

Status Description

Black

Not recommended for use because of lack of evidence of clinical effectiveness, cost effectiveness or safety.
Drugs which have been evaluated and rejected by the Medicines Value Group (MVG)
Drugs defined as ‘Low Priority’ by the South Central Priorities Committee
New drugs which have not as yet been evaluated by the MVG
Any drug not listed in the Buckinghamshire Formulary  

Red

Drugs which should only be prescribed in secondary care by a specialist.
Require specialist knowledge and/or equipment for patient selection and initiation
Require long term on-going monitoring and dose adjustment to ensure efficacy and minimise toxicity by a specialist
Designated as “hospital only“ by product licence, NICE, DoH or BNF
May need further evaluation by a specialist
Are hospital initiated clinical trial materials  

Red Specialist Centre

As for ‘red’ (above) with the addition of the following:
Designated by NHS England to require initiation by or in prior agreement with a Specialist Centre. Continuation where appropriate by BHT (or other secondary care provider)
Detailed requirements for Specialist Centre initiation to be described in the wording of the formulary restriction  

Amber Protocol

Drugs which should be initiated in secondary care by the specialist with follow-on prescription and monitoring according to a drug specific Shared Care Protocol(SCP). Prescribing may be continued in primary care following the SCP
Require specialist knowledge and/or equipment for patient selection and initiation
Require short or medium term (eg. 3 to 6 months) specialist monitoring of efficacy or toxicity. The need for stabilisation will vary with different drugs and patients, but is usually a minimum of 2 months (see principles for shared care)
Require significant long term monitoring
Require ongoing communication between the GP and the specialist
Have clearly defined consultant, GP and patient responsibilities documented in a shared care protocol (see responsibilities for amber protocol drugs)  

Amber Initiation

Drugs suitable for primary care prescribing following specialist initiation
Require specialist knowledge and/or equipment for patient selection
Monitoring does not require specialist knowledge or equipment
If the drug is one with which the primary care prescriber is unfamiliar the specialist is expected to provide sufficient information on the drug indication, dose, duration , monitoring and any further necessary dose adjustments
Require the first prescription to be written by the specialist  

Amber Recommended

Drugs suitable for primary care prescribing following specialist recommendation
As for amber initiation except that:- The first prescription may be written by the GP after specialist recommendation.  

Green

Drugs for which primary care prescribers would normally take full responsibility for prescribing and monitoring
Drugs not included in the Traffic Light list but included on joint formulary.
New drugs classified as red or amber but as greater experience regarding their safety and efficacy is established may move to Green after re-consideration by the MVG and APC.  

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